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Endothelial Dysfunction Is Implicated in PAH Etiology and Progression |
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Dysfunction of the endothelin (ET) system may contribute to the etiology and progression of pulmonary arterial hypertension (PAH), a rapidly progressing and fatal condition of the cardiopulmonary system.1 The loss of endothelial cell integrity, whether through genetic predisposition or environmental damage, and the accompanying reduction in nitric oxide and increase in ET production, are all recognized parts of the disease process.2 |
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Elevated levels of ET have been reported in patients with PAH.1 The pulmonary vascular endothelium produces excess ET in patients with PAH, particularly at the site of the most significant pathology, suggesting that local production of ET may contribute to disease development and progression.3,4 |
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Pulmonary Arterial Hypertension |
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Clinical Definition1 |
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Mean pulmonary arterial pressure >25 mm Hg |
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Pulmonary capillary wedge pressure ≤15 mm Hg |
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Pulmonary vascular resistance >3 Wood units |
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REFERENCES |
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1. |
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573–1619. |
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2. |
Gaine S. Pulmonary hypertension. JAMA. 2000;284:3160–3168. |
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Miyauchi T, Masaki T. Pathophysiology of endothelin in the cardiovascular system. Annu Rev Physiol. 1999;61:391–415. |
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Rich S, McLaughlin VV. Pulmonary hypertension. In: Libby P, Bonow RO, Mann DL, Zipes DE. Braunwald's Heart Disease. 2 vols. 8th ed. Philadelphia, PA: Saunders Elsevier; 2008:1883–1914. |
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5. |
Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993;328:1732–1739. |
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